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Dissecting Cell-Autonomous Function of Fragile X Mental Retardation Protein in an Auditory Circuit by In Ovo Electroporation
Published on: July 6, 2022
Fragile X-related protein family: a double-edged sword in neurodevelopmental disorders and cancer
Mrinmoyee Majumder1, Roger H Johnson2, Viswanathan Palanisamy1
1Department of Biochemistry and Molecular Biology, School of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Abstract:
The fragile X-related (FXR) family proteins FMRP, FXR1, and FXR2 are RNA binding proteins that play a critical role in RNA metabolism, neuronal plasticity, and muscle development. These proteins share significant homology in their protein domains, which are functionally and structurally similar to each other. FXR family members are known to play an essential role in causing fragile X mental retardation syndrome (FXS), the most common genetic form of autism spectrum disorder. Recent advances in our understanding of this family of proteins have occurred in tandem with discoveries of great importance to neurological disorders and cancer biology via the identification of their novel RNA and protein targets. Herein, we review the FXR family of proteins as they pertain to FXS, other mental illnesses, and cancer. We emphasize recent findings and analyses that suggest contrasting functions of this protein family in FXS and tumorigenesis based on their expression patterns in human tissues. Finally, we discuss current gaps in our knowledge regarding the FXR protein family and their role in FXS and cancer and suggest future studies to facilitate bench to bedside translation of the findings.
Insights
Fragile X-related (FXR) proteins are crucial for RNA metabolism and development. Their roles in fragile X syndrome, mental illness, and cancer reveal contrasting functions based on tissue expression, requiring further study.
Area of Science:
- Molecular Biology
- Neuroscience
- Oncology
Background:
- The fragile X-related (FXR) protein family, including FMRP, FXR1, and FXR2, are homologous RNA-binding proteins vital for RNA metabolism, neuronal plasticity, and muscle development.
- FXR proteins are implicated in fragile X mental retardation syndrome (FXS), the most common genetic cause of autism spectrum disorder.
Purpose of the Study:
- To review the FXR protein family's role in FXS, other mental illnesses, and cancer.
- To highlight recent findings on novel RNA and protein targets and their implications for neurological disorders and cancer biology.
- To discuss contrasting functions of FXR proteins in FXS and tumorigenesis based on human tissue expression patterns.
Main Methods:
- Review of existing literature and recent findings on FXR protein family.
- Analysis of FXR protein expression patterns in human tissues.
- Identification of novel RNA and protein targets associated with FXR proteins.
Main Results:
- FXR proteins play a critical role in FXS and are implicated in other mental illnesses and cancer.
- Recent discoveries have identified novel RNA and protein targets, advancing understanding of neurological disorders and cancer biology.
- Expression patterns in human tissues suggest contrasting roles for FXR proteins in FXS and tumorigenesis.
Conclusions:
- The FXR protein family has complex and potentially contrasting roles in FXS and cancer.
- Further research is needed to address knowledge gaps regarding FXR proteins in FXS and cancer.
- Future studies aim to translate findings from basic research to clinical applications for patients.
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10:59Generation and Characterization of Human Induced Pluripotent Stem Cell-derived Astrocytes Lacking Fragile X Messenger Ribonucleoprotein
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